Surgical Fixation Using Screw-Rod Construct Instrumentation for Upper Cervical Instability in Pediatric Down Syndrome Patients

Abstract

Study design: Retrospective case series.

Objectives: To describe the indications and outcomes of cervical fixation using modern instrumentation in a case series of pediatric Down syndrome (DS) patients.

Summary of background data: Cervical instability is the major cervical spine concern in children with DS. Although fixation techniques have advanced over the past quarter-century, the outcome of fixation with modern instrumentation for upper cervical instability in DS patients has not been thoroughly investigated.

Methods: We searched the orthopedic database at our institution for patients with a diagnosis of DS who had undergone a cervical spine fusion between 2006 and 2017. Patient demographics, diagnoses, surgical indications, surgical details, and complications were recorded. Preoperative imaging was reviewed to determine atlanto-dens intervals and spinal cord signal changes. Postoperative radiographs or CT scans were reviewed to determine union.

Results: Twelve DS patients met our inclusion criteria. The mean age at surgery was 9.3 years (range 3.8-18.8 years). Patients with secondary causes of instability included 7 patients with os odontoideum and 1 patient with a pars fracture. Three patients (25%) were identified on asymptomatic screening, with none of these having cord signal changes on magnetic resonance imaging (MRI). Modern implants (screws, plates, cages) were used in every patient in our series. The mean number of levels fused was 1.9 (range 1-5). The overall complication rate was 41.7% (5/12). Four patients required repeat surgery for nonunion. All patients with adequate radiographic follow-up demonstrated union (11/11, 100%). One patient was lost to follow-up.

Conclusions: Fixation for cervical instability is a critical component of the management of DS. A minority of patients receiving surgery were identified through asymptomatic screening. There was a high complication risk associated with surgery in our study; however, the addition of rigid fixation has lessened the complication rate compared with previous studies.

Level of evidence: Level IV.

Keywords: Cervical instability; Cervical spine; Down syndrome; Outcomes; Surgical fixation.